Biomedical subjects
Ola H Skjeldal
Publications and source records attributed to Ola H Skjeldal.
[Infantile spasms].
BACKGROUND: Infantile spasms are an epileptic manifestation typical of infancy. The spasms may be associated with a wide spectrum of brain abnormalities and diseases, but coexisting pathology is not always found. Prognosis depends to a substantial extent on the underlying condition. Most of the traditional antiepileptics are not effective. Adrenocorticotropic hormone (ACTH) and more recently vigabatrin are the most common first-line medications. MATERIAL AND METHODS: We have performed a literature search mainly in the PubMed database on the treatment of infantile spasms. The results are discussed in relation to Norwegian practice, for which the Norwegian Paediatric Society's recommendations are the main guidelines. RESULTS/INTERPRETATION: Vigabatrin has emerged as an accepted first-line medication for infantile spasms in Norway and many other countries, although it has not been shown that this therapy in general is as effective as ACTH/steroids. Well-designed treatment studies have been difficult to carry out. Until the final results of such ongoing studies should give different signals, it seems reasonable to continue to give vigabatrin before hormones. Oral steroids can probably be tried before ACTH, although a firm conclusion about this has yet to be drawn.
[Treatment of patients with neuromuscular disease in a warm climate].
BACKGROUND: Several patient groups request treatment in a warm climate, in spite of the fact that the effects of such treatment are undocumented. MATERIAL AND METHODS: 47 children and 40 adults with neuromuscular diseases were recruited, stratified according to sex, use or non-use of electric wheelchair, primary myopathy or hereditary neuropathy, and randomised into two adult and two children groups. The patients were treated in a rehabilitation centre, either on Lanzarote or in Norway. All patients were monitored with physical tests and questionnaires at the start of the study, at the end of the treatment period, after three months (all groups) and after six months (adults only). RESULTS: No significant differences in effect between the groups were found. In the warm climate, the adult patient group showed a statistically significant improvement regarding pain, quality of life, depression, and results of physical tests at the end of treatment. After three months, the improvement in physical tests was still present. Among adult patients treated in Norway, improvement in physical tests was statistically significant after three months, but not at the end of the treatment period. INTERPRETATION: This study did not show a statistically significant difference between patients with various neuromuscular diseases treated in a warm climate compared to similar patients treated in Norway.
[Habilitation of children with cerebral palsy].
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Succinate in dystrophic white matter: a proton magnetic resonance spectroscopy finding characteristic for complex II deficiency.
A deficiency of succinate dehydrogenase is a rare cause of mitochondrial encephalomyopathy. Three patients, 2 sisters and 1 boy from an unrelated family, presented with symptoms and magnetic resonance imaging signs of leukoencephalopathy. Localized proton magnetic resonance spectroscopy indicated a prominent singlet at 2.40ppm in cerebral and cerebellar white matter not present in gray matter or basal ganglia. The signal was also elevated in cerebrospinal fluid and could be identified as originating from the two equivalent methylene groups of succinate. Subsequently, an isolated deficiency of complex II (succinate:ubiquinone oxidoreductase) was demonstrated in 2 patients in muscle and fibroblasts. One of the sisters died at the age of 18 months. Postmortem examination showed the neuropathological characteristics of Leigh syndrome. Her younger sister, now 12 months old, is also severely affected; the boy, now 6 years old, follows a milder, fluctuating clinical course. Magnetic resonance spectroscopy provides a characteristic pattern in succinate dehydrogenase deficiency.